gct.ppt s babu

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GIANT CELL TUMOUR (G C T) Dr S. BABU MBBS,D ortho :

GIANT CELL TUMOUR (G C T) Dr S. BABU MBBS,D ortho

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Giant cell tumor of bone first was described in 1818 by Cooper and Travers Brown or myeloid tumor called by Paget. Osteoclastoma called by British. Its local aggressiveness was described by Nelaton. Its malignant potential by Virchow. Malignant GCT called as giant-cell fibrosarcoma by Agerter.

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Giant cell tumor (GCT) is a locally aggressive osteolytic tumor characterized by richly vascularized tissue containing proliferating mononuclear stromal cells and many osteoclast -like, multinucleated large giant cells (characteristic) randomly but evenly distributed throughout .

CELL OF ORIGIN:

CELL OF ORIGIN Giant cell tumours are believed to have monocyte –macrophage lineage and form via fusion of mononuclear cells Apart from multinucleated giant cells ,there are two mononuclear cell types 1.first one has round morphology and resembles monocytes 2.second cell type is spindle shaped fibroblast like stromal cells

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cell culture experiments revealed the stromal cells to be the proliferating component,the other two cell types,monocyte and multinucleated giant cells were lost after few cell culture passages Latest results reveal that stromal cells secrete a variety of cytokines and differentiation factors like MCP1,ODF &M-CSF These molecules are monocyte chemo attractants and are essential for osteoclast differentiation,suggesting that the stromal cell stimulates blood monocytes immigration into tumour tissue and enhances their fusion into osteoclast like ,multi nucleated giant cells that resemble a normal osteoclast that is able to resorb bone that leads to extended osteolysis

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SYNONYMS myeloid sarcoma tumor of myeloplexus osteoblastoclastoma osteoclastoma

Incidence:

Incidence Constitute 4 - 9.5% (~5%) of bone tumors 18 – 23% benign bone neoplasms 0.4 – 1.2% of tumors are malignant 10% primary 90% secondary (radiation) High incidence in china (SEAR) Noted to be common in some parts of andhra pradesh

Clinical Presentation :

Clinical Presentation GCT occurs exclusively after skeletal maturity between the ages of 20 and 40 years female preponderance 3:2 (in India male pred). arise in long bones and extend to the articular end presentation in the metaphysis are seen in skeletally immature patients .

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common skeletal sites are >75% are situated near articular end of tubular long bone 55% situated around knee jt the distal femur, the proximal tibia, the distal radius(white giant cell tumor-10%), proximal end of the fibula the proximal humerus(6%), and the sacrum(female preponderance) .

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Rarely in other sites, such as the bones of hands and feet(2%), the vertebral bodies, the ribs the scapula , the ischium and the skull Very uncommonly, GCT may be multifocal : frequency of 0.04% to 1% (goltz syndrome) Multiple leisions associated with Pagets disease

Clinical features:

Clinical features SYMPTOMS & SIGNS 1.Pain: chronic progressive dull aching, worse at night and with activity increased 2.Swelling :end of the long bone expands to one side ,over lying skin is stretched,no dilated vessels (contrast with osteosarcoma),pressure over the swelling produces audible &palpable crackling as the cortex is ruptured (eggshell crackling)

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3.Tenderness over the swelling is moderate or absent 4.Limitation of jt motion: not observed until late ,as the tumour expands and distorts the end of the bone No increase of joint fluid joint is not invaded by tumour 5.pathological #: occurs late (large extent of cancellous &cortical bone is destroyed)

Gross and microscopic appearance :

Gross and microscopic appearance

Investigations:

Investigations Radiograph *large , circumscribed area of decreased density asymmetrically located in the epiphysis ,begins subcortically & extends towards metaphysis .cortex is expanded outwards &thinned ,no periosteal new bone formation ,characteristic soap bubble appearace (multiple septae of bone & soft tissue traverse the interior )

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Pure lytic lesion (70% of the cases). * Geographic type of bone destruction Multiloculated lesion with characteristic appearance ( honeycomb-like pattern) (30% of the cases) .* The lesion is radiolucent, frequently expansive, and eccentrically located , without marginal sclerosis but usually with well-defined borders

X-ray:

X-ray

Radiological grading (campanacci):

Radiological grading (campanacci) Grade1:well marginated border of a thin rim of mature bone cortex intact , slightly thinned but not deformed Grade2:well defined margin but no radioopaque rim cortex and rim of reactive bone are thin &moderately expanded but still present Grade 3:rapid &permeative growth tumour bulges into soft tissue with fuzzy border not limited by apparent shell of reactive bone

Other investigations:

Other investigations * CT scan or MRI ----- particularly where soft-tissue or intra-articular extension is suspected. * Chest x-ray- ----- GCT has the potential for metastasis to the lungs : * Phosphocalcic assessment to confirm the absence of bone lesion of hyperparathyroidism

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CT scan MRI-GCT radius

BONE SCAN:

BONE SCAN Limited importance Extended uptake is noted beyond the actual iesion due to regional hyperemia Overestimating the actual size

Differential diagnosis :

Differential diagnosis 1.Aneurysmal bone cyst .metaphyseoepiphyseal blow-out .thinned out cortices 2.Brown tumour .eccentric skeletal demineralisation .sub periosteal bone resorption .sr.calcium >10 mg /dl

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3.Non ossifying fibroma .first decade of age .meta physeal location .excentric oval defects 4.Unicameral bone cyst .first 2 decades .metaphyseal location .proximal humerus& femur-MC .cyst filled with clear fluid .cyst wall has fibrous tissue

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5.Fibrous dysplasia .first two decades .metaphyseal location .proximal femur-MC .ground glass appearance 6.Chondroblastoma .second decade .epiphyseal location .radiolucent with patchy opacities .multiple pinpoint calcifications

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7.Giant cell reperative granuloma .second &third decade .mandible –MC .maxilla .radiolucent focus 8.Ossefying fibroma .second&third decade .maxilla & mandible .radio opaque .bony hard tissue

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9.Sub periosteal giant cell tumour .rapid development after trauma of an osteolytic lesion containing blood .giant cells seen in ossifying stroma of fibrous tissue .no expansion of cortex .spontaneous healing &reossification

Staging(Enneking):

Staging(Enneking) stage I: - benign latent giant cell tumors; - no local agressive activity; stage II: - benign active GCT; - imaging studies demonstrate alteration of the cortical bone structure; stage III: - locally aggressive tumors; - imaging studies demonstrate a lytic lesion surrounding medullary and cortical bone; - there may be indication of tumor penetration through the cortex into the soft tissues

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Biopsy of a GCT Does not involve "removing a small tissue sample of the tumor". A large-size biopsy specimen (almost the entire contents of the cavity) is necessary. considered as the first step of the curettage/bone-grafting procedure.

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GCT may have both benign and malignant areas. A large-size biopsy specimen is required to avoid missing any malignant areas. *Petite biopsie = PIEGE = Small biopsy specimen = PITFALL *Biopsie large = SECURITE = Large biopsy specimen = SAFETY

Histopathology :

Histopathology include a dual population of fibrocytic or monocytic mononuclear stromal cells and of giant cells that are usually uniformly distributed throughout the tumor The giant cells resemble osteoclasts exhibit marked acid phosphatase activity They are much larger than normal osteoclasts and are not apposed to bone surfaces and therefore do not possess a ruffled border . Areas of hemorrhage, and groups of foam cells and of hemosiderin-laden macrophages, are frequently present

Histologic classification of GCT:

Histologic classification of GCT Three grades , corresponding to the number and size of giant cells and the degree of pleomorphism of the mononuclear cells Grade I tumor dense layer of huge giant cells with up to 100 or more nuclei, almost covering the tumor tissue proper of mononuclear cells. Grade II tumor exhibits a considerable reduction in the size and number of giant cells, and the mononuclear cells may be pleomorphic to some degree. Grade III tumors , the giant cells are further reduced in number and there is an increase in pleomorphic mononuclear cells.

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Histologically 3 grades (JAFFE) grade 1 : benign, low aggressive behavior grade 2 : benign, highly aggressive behavior grade 3 : malignant

5 E’s IN G C T:

5 E’s IN G C T EPIPHYSEO-METAPHYSEAL ORIGIN EGG-SHELL CRACKLING ECCENTRICALLY PLACED EXPANSILE EXCISION

Malignant Giant Cell Tumor :

Malignant Giant Cell Tumor Three types: (a) primary , in which the tumor was malignant from the onset (de novo). (b) evolutionary, in which there was malignant transformation of originally benign GCT spontaneously or after multiple surgical resections for recurrent lesions, or after a long latency period. (c) secondary, developing as a result of malignant transformation of an initially benign tumor after radiation therapy.

Treatment Strategies:

Treatment Strategies 1. curettage 2. curettage and cytotoxic agents such as 5%phenol,zinc chloride,70% alcohol, and H 2 O 2. 3. curettage and a physical adjuvant (polymethylmethacrylate,liquid nitrogen and cryosurgery) 4. curettage & bonegrafting 5. radical resection. 6. radiation therapy 7. amputation 8. embolization--------- in unresectable tumors.

Appropriate method for an effective GCT curettage:

Appropriate method for an effective GCT curettage performed with a large curette--- remove macroscopically the main part of the tumor Then, decrease the size of the curette to the smallest size (so-called "grain de mil") patiently scrape the tumor remnants off the bone walls, paying attention to crevices, tiny recesses and pockets, fissures ....

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the curettage material is soft and dark brown (very characteristic Buff color )

Method --- currently using :

Method --- currently using After through CURETTAGE Carbonization of walls : it is achieved with an electrocautery and a ball-tipped probe, using high current intensity . The color of the walls turns black, a second curettage is performed, followed by jet lavage. The procedure is repeated 2 or 3 times .

Carbonization of walls :

Carbonization of walls

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Motorized burr and cautery are used to complete and the excision

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Phenol, which coagulates all proteinaceous substances, may remove microscopic tumor residua that remains after curettage.

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polymethylmethacrylate was used when simple filling with autologous bone was insufficient Heat of polymerization of the polymethylmethacrylate could induce tumor necrosis and advance the excision margin after curettage. Moreover, the monomer has a direct toxic effect that results in hypoxia.

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Cryotherapy 1. Liquid nitrogen 2. Argon

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Cryosurgery is recommended as a physical adjuvant to curettage in the treatment of giant cell tumor of bone. It extends the margin of a simple curettage or resection curettage and makes it biologically equivalent to that of a wide resection .

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Intralesional bisphosphonate therapy. Zolendronate, and pamidronate, have been demonstrated to cause apoptosis of giant cells.

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At the completion of the procedure the window is replaced with bone graft or with a bone graft substitute

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At the completion of the procedure, the window is replaced with bone graft or bone graft substitute

Filling options available :

Filling options available Autologous bone grafts Morsellized femoral head bone grafts from the bone bank. Mix of autograft and allograft Bone cement

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The advantage of bone graft is It is biologic. bone stock is restored. This can be important in the situation of a patient who develops late osteoarthritis as a more conventional total knee arthroplasty can then be performed. Bone graft may also be advantageous to promote healing in the setting of pathologic fracture

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The cavity is packed densely with graft material using a graft pusher

Disadvantages of cement :

Disadvantages of cement compromise healing of a fracture block of bone cement was almost flush with the joint line. Such a thin osteocartilaginous shell may wear out or fracture.

Indications of cement :

Indications of cement In the elderly. In multiply operated patients who have previously undergone multiple bone graft harvests. GCT infection secondary to biopsy (advantage of using antibiotic bone cement). In cases where there is no subchondral extension of the tumor (with at least 1 cm of healthy bone left between the tumor and the joint line

Treatment:

Treatment Stage 1 or 2 Lesions - intra-lesional excision is treatment of choice excision is facilitated by making a large cortical window window must be large enough to allow complete access to every corner of the intra-osseous lesion; - high speed burr is used to complete the excision, with care taken to remove 5 mm of normal bone surrounding the lesion;

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Recurrent or Stage 3 Lesions: - this category includes major pathologic frx, articular or cortical penetration by the tumor; - en bloc excision w/ wide margin along w/ appropriate reconstruction; - en bloc excision often involves removal of one side the joint, necessitating a major limb reconstructive procedure

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In areas such as the distal ulna or fibula, en bloc excision is preferred over currettage The treatment of sacral giant-cell tumours by serial arterial embolisation

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Custom mega prosthesis for juxta articular giant cell tumour

Indications:

Indications It is the primary modality in the management of malignant bone tumors of lower limb. The use of mega prosthesis has become the method of choice after bone tumor resection at the knee.

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The design being used is the prosthesis with thrust-bearing pad and rotating axis mechanism . The basic components of the prosthesis are a femoral shaft, a condylar component, a median component, a thrust-bearing pad, a pivot pin, collar bushes and tibial component.

Custom mega prosthesis:

Custom mega prosthesis

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Proximally, the prosthesis is angulated laterally by 6° to resemble the anatomical axis of the lower limb . The function of the thrust-bearing pad is to impart a flexion of 150° between the femoral and tibial components.

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The ultra-high-molecular-weight polyethylene-bearing pad serves to relocate the load transmitted during weight-bearing. The rotating axis mechanism provides 3° of rotation between the femoral and tibial component

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Advantages Early resumption of knee function with unassisted ambulation and Cost-effectiveness Least rates of recurrence. Complications Flap necrosis Secondary infection Aseptic loosening fracture and breakage

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thank you

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